Functional Analysis of Polymorphonuclear Leukocytes in Siblings of Glucose-6-Phosphate Dehydrogenase Deficiency

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Title ( eng )
Functional Analysis of Polymorphonuclear Leukocytes in Siblings of Glucose-6-Phosphate Dehydrogenase Deficiency
Title ( jpn )
ブドウ糖6リン酸脱水素酵素欠損症同胞例の多核白血球機能
Creator
Matsuura Ryoji
Kishi Takamasa
Okahata Hiroyasu
Tanabe Akio
Sakura Nobuo
Sawano Kunihiko
Usui Tomofusa
Source Title
Hiroshima Journal of Medical Sciences
Volume 32
Issue 2
Start Page 173
End Page 178
Journal Identifire
[PISSN] 0018-2052
[EISSN] 2433-7668
[NCID] AA00664312
Abstract
Several functions of polymorphonuclear leukocytes (PMNL) were examined in two siblings with glucose-6-phosphate dehydrogenase (G6PD) deficiency. In spite of marked depression of G6PD activity of PMNL, the patients had no susceptibility to bacterial infections. Qualitative nitroblue tetrazolium (NET) reduction was normal in both cases. Quantitative NBT reduction was normal in one case and subnormal in the other. Bactericidal activity also was normal. However, superoxide anion (O2-) release, oxygen (O2) consumption and chemiluminescence (CL) response were significantly decreased in both cases. This dysfunction was more marked when glucose was added extracellularly. It is concluded that these tests are more suitable than the NBT test for the screening of PMNL G6PD deficiency. It is also considered that there is a large reserve supply of active oxygens necessary for normal bactericidal activity of PMNL.
Keywords
Glucose-6-phosphate dehydrogenase
Polymorphonuclear leukocytes
Chronic granulomatous disease
Descriptions
This work has been supported in part by Grants-in-Aid for Scientific Research (Project Nos. 544050, 448227) from the Ministry of Education, Scientific and Culture of Japan and a Grant-in-Aid from the Japan Medical Research Foundation.
NDC
Medical sciences [ 490 ]
Language
eng
Resource Type departmental bulletin paper
Publisher
Hiroshima University School of Medicine
Date of Issued 1983-06
Publish Type Version of Record
Access Rights open access
Source Identifier
[ISSN] 0018-2052
[NCID] AA00664312
[PMID] 6618900